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Müllerian Duct Cysts
MULLERIAN DUCT CYSTS CLYDE L. DEMING
AND
ROBERT R. BERNEIKE
From the Department of Surgery, Section on Urology, Yale University, and the New Haven Hospital, New Haven, Connecticut
Cysts of the deep male J:Jelvis fall into five general groups: 1. Those arising from the Wolffian body or its ducts. 2. Those arising from the Mullerian ducts. 3. Those which result from an obstruction of the normal ducts by enlargement of the prostatic utricle. 4. Those of the seminal vesicle proper, thought to be due to occlusion of a diverticulum. 5. Those of inflammatory or parasitic origin, as, for instance, hydatid cysts of the septum between the bladder, rectum and prostate, and other miscellaneous cystic tumors such as dermoids, aneurysms and degenerative cysts of hyperplastic or malignant prostates. Coppridge, in 1939, presented an excellent review of the literature, from which he reports 6 cases of massive midline cysts simulating a distended bladder and originating deep in the tissues between the bladder and the rectum. Many features of these cases suggest that they were probably Miillerian duct cysts. In addition, he reports a case of his own, which was undoubtedly a cyst arising from a remnant of Muller's duct. Hennessey, in the same year, added another case of a large, symmetrical midline cyst which satisfies the criteria for Mullerian duct cysts. Hallock, in 1931, reported an orange-sized cyst, palpable rectally just above the prostate, and presumably a cyst of Muller's duct; and R. Campbell Begg, in 1936, added another to the yet small list of authenticated cases. It is the purpose of this paper to present another case of a cyst of Muller's duct in a male and to outline a group of diagnostic criteria to silhouette the disease. There has been a tendency in the literature of prostatic disease to include Miillerian duct cysts in a loosely-defined category of prostatic cysts, the ultimate differentiation depending on the surgical and pathological findings. Emmett and Braasch, in a review of 22 cases of prostatic cysts, described cysts either of one of the lobes of the prostate or of the whole prostate itself. Most of these, when viewed cystoscopically, were small cystic structures projecting into the bladder or posterior urethra. Some were palpated rectally as cystic masses in various parts of the prostatic gland. In Emmett and Braasch's series of cases, the cystic mass is described as extending upward between the lateral lobes of the prostate only in the case of Hallock, mentioned above. The rectal palpation of a relatively large cystic mass which does not directly involve or originate in the prostatic tissue but seems rather to arise from an area between the lobes of a normal prostate is in keeping with one of the long-established criteria associated with the postoperative diagnosis of Mullerian duct cyst. Furthermore, true prostatic cysts have rarely attained any great size, the vast majority being of the order of magnitude of a walnut or smaller. In analysis, then, of the available reports of cases which are proved or even probable examples of Miillerian duct cysts, several common features are promi563
564
C. L. DEMING AND R. R. BERNEIKE
nent. Except for R. Campbell Begg's patient, who was 57 years of age, all have been between the ages of 19 and 40. All of the tumors were symmetrical and situated in the midline. Most of them have been of fairly large size and were palpable abdominally. None have contained spermatozoa, and the descriptions of the cyst fluid suggests that it consisted in all cases of various stages of degeneration of blood, ranging from "dark brown" and "brownish green" to frankly bloody fluid. When operative removal was attempted, it was found in all but Hallock's case that the anterior wall of the cyst and the posterior wall of the bladder were practically one and the same structure, making complete removal extremely difficult and, in many cases, impossible. The several clinical features characteristic of Miillerian duct cysts are: 1. A symmetrical cystic mass is palpable rectally just above the prostate in the midline. 2. The prostate is normal or, at least, not directly involved in the cystic process. 3. Urethrocystoscopy shmvs only encroachment on the bladder lumen by an extrinsic mass and perhaps symmetrical lateral displacement of both ureters. The utricle may or may not be slightly enlarged, and bloody discharge may be encountered at its orifice. 4. Fluid from the cyst does not show any spermatozoa or parasites and probably shows only old changed blood. 5. Skin reaction to echinococcus antigen is negative. Histologically and pathologically, there are other features which are s11id to characterize all Miillerian duct cysts. First, the epithelium should be cuboidal or low columnar in type. Second, there should be evidence of cystadenoma. Third, a cord of tumor tissue should pass from the cyst through the prostate to the region of the verumontanum without affecting the adjacent prostatic tissue. The differential diagnosis is briefly tabulated as follows: 1. Prostatic cyst: a. Palpable in prostatic tissue or visualized cystoscopically as projecting into the posterior urethra or bladder. b. Almost invariably small. c. Usually associated with other prostatic disease. 2. Seminal vesicle cyst: a. Prostate usually normal. b. Cyst usually laterally situated. c. Size variable; may be very large. d. Frequently contains spermatozoa. 3. Miillerian duct cyst: a. Prostate usually normal. b. Cyst always in midline. c. Usually quite large. cl. Does not contain spermatozoa.
Miillerian duct cysts have been treated conservatively by aspiration or radically by excision. The recent literature favors complete removal of the cyst. The most frequently successful approach has been suprapubic, since most of these cysts extend well up along the posterior wall of the bladder. Infrequently, the combined abdomino-perineal approach may be indicated. CASE REPORT
A. G., New Haven Hospital Unit No. B-19092, a 30-year old, single, Italian male, born and raised in Connecticut, was admitted to the New Haven Hospital
MULLERIAN DUCT CYSTS
565
October 15, 1942, with the chief complaints of bloody urethral discharge, urinary frequency, and watery discharge from the rectum. Approximately 2 years prior to admission, the patient first noticed "flecks" of blood in his urine, which had persisted at approximately monthly intervals. For about the same duration and at similar intervals he had noticed small blood stains on his underclothing and pajamas, apparently coming from the urethra. During the year and a half preceding admission he had experienced gradually increasing frequency of urination both by day and by night. Accompanying the latter complaint was moderate difficulty in starting and stopping the stream, associated ,,ith a shooting pain originating in the perineum and radiating down the ventrum of the penis. For 3 or 4 months there had been an occasional, scanty, "watery" discharge from the rectum with no other remarkable gastro-intestinal symptoms. The only sexual complaint was that overindulgence seemed to aggravate the frequency and to cause some burning on urination. Intercourse was apparently satisfactory. Venereal history was denied specifically and symptomatically. Vital signs were normal. General physical examination revealed a rather poorly nourished white male ·whose only abnormal physical findings were limited to the genitalia and rectum. Penis, testes and vasa were normal to palpation, but both epididymes were thickened and slightly enlarged, more so on the left. Rectally, the prostate ,vas normal in size, shape, and consistency; but the lateral lobes were somewhat separated near the upper edge, and from between them there extended upward a tense, cystic mass, which was roughly in the midline but perhaps slightly larger on the right than on the left. The upper limits of the cyst could not be reached, nor could it be palpated suprapubically, even on bimanual examination. Blood counts and smear showed only leukocytosis of 13,400, with an essentially normal differential count. A centrifuged urine sediment shmved 2 to 4 red blood cells per high power field, but the urine was otherwise negative. Kahn test was negative. Non-protein nitrogen was 26 mg per cent, and the phthalein test yielded 75 per cent in 2 hours. Stool examination was negative. The cyst was tapped with a lumbar puncture needle introduced through the perineum, and about 6 cc of watery, chocolate-colored, odorless fluid were withdrawn. Eight cubic centimeters of 40 per cent Skiodan were then injected into the cyst, and films of this simultaneously ,,ith retrograde pyelograms showed a pear-shaped retrovesical mass extending up to the dome of the bladder and roughly in the midline, although somewhat larger on the right (figs. 1, 2, 3). Microscopic examination of the cyst fluid showed only rare laked red blood cells, a few white blood cells, and considerable amorphous material. No microorganisms were seen. Culture was sterile. Cystoscopy showed an appreciable elevation of the trigone and base of the bladder, but the posterior urethra and verumontanum showed only moderate vascular engorgement. The ureters were easily catheterized, and retrograde pyeloureterograms sho,ved nothing abnormal. Function was good in both kidneys, and there was no infection. Urethrogram showed a normal urethra without evidence of diverticulum. Barium enema revealed no abnormal findings.
566
C. L, DEMING AND R. R. BERNEIKE
Operation:-Ethylene-cyclopropane anaesthesia was used. Through a midline suprapubic incision the posterior wall of the bladder was exposed by stripping away the peritoneum. Adherent to the lower two-thirds of the posterior wall
Fm. 1. Retrograde pyelogram and injection of Mullerian duct cyst
Fm. 2. Cystogram and injection of Mullerian duct cyst.
Antero-posterior view
of the bladder was a grayish-blue cystic mass about the aggregate size of three hen's eggs. This was dissected away from the bladder with considerable difficulty, and its attachment was traced down to the prostate. Attached like two
MULLERIAN DUC'r CYSTS
567
ears to the postero-lateral aspects of the cyst were the two seminal vesicles; and, at the same points, apparently extending into the cyst, were the two vasa deferentia. The stalk of the cyst was traced down to the prostatic urethra, where its
Fm. 3. Same as figure 2.
Lateral view
Fm. 4. Photograph of surgical specimen of Mullerian duct cyst showing ventral surface. V-vas; SY-seminal vesicle; P-prostate; C-connection of Mullerian duct cyst with posterior urethra.
attachment was divided. The cyst was removed in toto with most of the prostate, both seminal vesicles, and the distal 3 inches of the vasa deferentia (fig. 4). Pathological Report:~"The portion of the specimen representing the prostate
568
C. L. DEMING AND R. R. BERNEIKE
is traversed by a canal 7 mm. in diameter. This canal is continuous above with a large, somewhat flattened, oval-shaped cavity, the inner surface of which is grayish-white and composed of minute nodules and sacc11lations. No demonstrable connection exists between the lumen of the vasa and that of the cyst. Sections of prostate show the usual epithelial lining with ducts and acini. Sections of the large cyst wall itself show the wall to be made up of a thick layer of smooth muscle and fibrous connective tissue. It is partially lined by a flattened layer of deep blue staining epithelial cells. Diagnosis :~Mullerian duct cyst, seminal vesicles, and portions of prostate and vasa deferentia." The patient's convalescence was complicated by drainage of urine from the suprapubic wound for 4 days, but this responded satisfactorily to an inlying urethral catheter. A superficial wound infection was controlled by conservative measures, and the patient was discharged on the twenty-fourth postoperative day. The patient has been seen twice since he left the hospital, and he is free of urinary symptoms. Rectal examination two months after the patient's discharge from the hospital was normal except that in the region of the prostate there was palpable a mass of indurated tissue about two-thirds the size of the normal prostate. CONCLUSION
An attempt has been made to establish some clear clinical features diagnostic of Mullerian duct cyst. A detailed report of a case is presented.
789 Howard Ave., New Haven, Conn. REFERENCES BEGG, R. CAMPBELL: Massive cystadenoma of Muller's duct. Brit. J. Urol., 8: 1936. CoPPRIDGE, W. M.: Mi.illerian duct cysts. South. M. J., 32: 248-251, 1939. DEMING, CLYDE L.: Cyst of the seminal vesicle. Trans. Am. Assoc. Genito-Urinary Surg., 1935. EMMETT, JoHN L., AND BRAASCH, WILLIAM F.: Cysts of the prostate gland. J. Urol., 36: 236-249, 1936. HALLOCK, L. A.: Large hemorrhagic cyst of the prostate gland. Am. J. Cancer (Supp.) 15: 2331-2340, 1931. HENNESSEY, RusSELL A.: Mi.illerian duct cysts. J. Urol., 42: 1042-1050, 1939.
AND
ROBERT R. BERNEIKE
From the Department of Surgery, Section on Urology, Yale University, and the New Haven Hospital, New Haven, Connecticut
Cysts of the deep male J:Jelvis fall into five general groups: 1. Those arising from the Wolffian body or its ducts. 2. Those arising from the Mullerian ducts. 3. Those which result from an obstruction of the normal ducts by enlargement of the prostatic utricle. 4. Those of the seminal vesicle proper, thought to be due to occlusion of a diverticulum. 5. Those of inflammatory or parasitic origin, as, for instance, hydatid cysts of the septum between the bladder, rectum and prostate, and other miscellaneous cystic tumors such as dermoids, aneurysms and degenerative cysts of hyperplastic or malignant prostates. Coppridge, in 1939, presented an excellent review of the literature, from which he reports 6 cases of massive midline cysts simulating a distended bladder and originating deep in the tissues between the bladder and the rectum. Many features of these cases suggest that they were probably Miillerian duct cysts. In addition, he reports a case of his own, which was undoubtedly a cyst arising from a remnant of Muller's duct. Hennessey, in the same year, added another case of a large, symmetrical midline cyst which satisfies the criteria for Mullerian duct cysts. Hallock, in 1931, reported an orange-sized cyst, palpable rectally just above the prostate, and presumably a cyst of Muller's duct; and R. Campbell Begg, in 1936, added another to the yet small list of authenticated cases. It is the purpose of this paper to present another case of a cyst of Muller's duct in a male and to outline a group of diagnostic criteria to silhouette the disease. There has been a tendency in the literature of prostatic disease to include Miillerian duct cysts in a loosely-defined category of prostatic cysts, the ultimate differentiation depending on the surgical and pathological findings. Emmett and Braasch, in a review of 22 cases of prostatic cysts, described cysts either of one of the lobes of the prostate or of the whole prostate itself. Most of these, when viewed cystoscopically, were small cystic structures projecting into the bladder or posterior urethra. Some were palpated rectally as cystic masses in various parts of the prostatic gland. In Emmett and Braasch's series of cases, the cystic mass is described as extending upward between the lateral lobes of the prostate only in the case of Hallock, mentioned above. The rectal palpation of a relatively large cystic mass which does not directly involve or originate in the prostatic tissue but seems rather to arise from an area between the lobes of a normal prostate is in keeping with one of the long-established criteria associated with the postoperative diagnosis of Mullerian duct cyst. Furthermore, true prostatic cysts have rarely attained any great size, the vast majority being of the order of magnitude of a walnut or smaller. In analysis, then, of the available reports of cases which are proved or even probable examples of Miillerian duct cysts, several common features are promi563
564
C. L. DEMING AND R. R. BERNEIKE
nent. Except for R. Campbell Begg's patient, who was 57 years of age, all have been between the ages of 19 and 40. All of the tumors were symmetrical and situated in the midline. Most of them have been of fairly large size and were palpable abdominally. None have contained spermatozoa, and the descriptions of the cyst fluid suggests that it consisted in all cases of various stages of degeneration of blood, ranging from "dark brown" and "brownish green" to frankly bloody fluid. When operative removal was attempted, it was found in all but Hallock's case that the anterior wall of the cyst and the posterior wall of the bladder were practically one and the same structure, making complete removal extremely difficult and, in many cases, impossible. The several clinical features characteristic of Miillerian duct cysts are: 1. A symmetrical cystic mass is palpable rectally just above the prostate in the midline. 2. The prostate is normal or, at least, not directly involved in the cystic process. 3. Urethrocystoscopy shmvs only encroachment on the bladder lumen by an extrinsic mass and perhaps symmetrical lateral displacement of both ureters. The utricle may or may not be slightly enlarged, and bloody discharge may be encountered at its orifice. 4. Fluid from the cyst does not show any spermatozoa or parasites and probably shows only old changed blood. 5. Skin reaction to echinococcus antigen is negative. Histologically and pathologically, there are other features which are s11id to characterize all Miillerian duct cysts. First, the epithelium should be cuboidal or low columnar in type. Second, there should be evidence of cystadenoma. Third, a cord of tumor tissue should pass from the cyst through the prostate to the region of the verumontanum without affecting the adjacent prostatic tissue. The differential diagnosis is briefly tabulated as follows: 1. Prostatic cyst: a. Palpable in prostatic tissue or visualized cystoscopically as projecting into the posterior urethra or bladder. b. Almost invariably small. c. Usually associated with other prostatic disease. 2. Seminal vesicle cyst: a. Prostate usually normal. b. Cyst usually laterally situated. c. Size variable; may be very large. d. Frequently contains spermatozoa. 3. Miillerian duct cyst: a. Prostate usually normal. b. Cyst always in midline. c. Usually quite large. cl. Does not contain spermatozoa.
Miillerian duct cysts have been treated conservatively by aspiration or radically by excision. The recent literature favors complete removal of the cyst. The most frequently successful approach has been suprapubic, since most of these cysts extend well up along the posterior wall of the bladder. Infrequently, the combined abdomino-perineal approach may be indicated. CASE REPORT
A. G., New Haven Hospital Unit No. B-19092, a 30-year old, single, Italian male, born and raised in Connecticut, was admitted to the New Haven Hospital
MULLERIAN DUCT CYSTS
565
October 15, 1942, with the chief complaints of bloody urethral discharge, urinary frequency, and watery discharge from the rectum. Approximately 2 years prior to admission, the patient first noticed "flecks" of blood in his urine, which had persisted at approximately monthly intervals. For about the same duration and at similar intervals he had noticed small blood stains on his underclothing and pajamas, apparently coming from the urethra. During the year and a half preceding admission he had experienced gradually increasing frequency of urination both by day and by night. Accompanying the latter complaint was moderate difficulty in starting and stopping the stream, associated ,,ith a shooting pain originating in the perineum and radiating down the ventrum of the penis. For 3 or 4 months there had been an occasional, scanty, "watery" discharge from the rectum with no other remarkable gastro-intestinal symptoms. The only sexual complaint was that overindulgence seemed to aggravate the frequency and to cause some burning on urination. Intercourse was apparently satisfactory. Venereal history was denied specifically and symptomatically. Vital signs were normal. General physical examination revealed a rather poorly nourished white male ·whose only abnormal physical findings were limited to the genitalia and rectum. Penis, testes and vasa were normal to palpation, but both epididymes were thickened and slightly enlarged, more so on the left. Rectally, the prostate ,vas normal in size, shape, and consistency; but the lateral lobes were somewhat separated near the upper edge, and from between them there extended upward a tense, cystic mass, which was roughly in the midline but perhaps slightly larger on the right than on the left. The upper limits of the cyst could not be reached, nor could it be palpated suprapubically, even on bimanual examination. Blood counts and smear showed only leukocytosis of 13,400, with an essentially normal differential count. A centrifuged urine sediment shmved 2 to 4 red blood cells per high power field, but the urine was otherwise negative. Kahn test was negative. Non-protein nitrogen was 26 mg per cent, and the phthalein test yielded 75 per cent in 2 hours. Stool examination was negative. The cyst was tapped with a lumbar puncture needle introduced through the perineum, and about 6 cc of watery, chocolate-colored, odorless fluid were withdrawn. Eight cubic centimeters of 40 per cent Skiodan were then injected into the cyst, and films of this simultaneously ,,ith retrograde pyelograms showed a pear-shaped retrovesical mass extending up to the dome of the bladder and roughly in the midline, although somewhat larger on the right (figs. 1, 2, 3). Microscopic examination of the cyst fluid showed only rare laked red blood cells, a few white blood cells, and considerable amorphous material. No microorganisms were seen. Culture was sterile. Cystoscopy showed an appreciable elevation of the trigone and base of the bladder, but the posterior urethra and verumontanum showed only moderate vascular engorgement. The ureters were easily catheterized, and retrograde pyeloureterograms sho,ved nothing abnormal. Function was good in both kidneys, and there was no infection. Urethrogram showed a normal urethra without evidence of diverticulum. Barium enema revealed no abnormal findings.
566
C. L, DEMING AND R. R. BERNEIKE
Operation:-Ethylene-cyclopropane anaesthesia was used. Through a midline suprapubic incision the posterior wall of the bladder was exposed by stripping away the peritoneum. Adherent to the lower two-thirds of the posterior wall
Fm. 1. Retrograde pyelogram and injection of Mullerian duct cyst
Fm. 2. Cystogram and injection of Mullerian duct cyst.
Antero-posterior view
of the bladder was a grayish-blue cystic mass about the aggregate size of three hen's eggs. This was dissected away from the bladder with considerable difficulty, and its attachment was traced down to the prostate. Attached like two
MULLERIAN DUC'r CYSTS
567
ears to the postero-lateral aspects of the cyst were the two seminal vesicles; and, at the same points, apparently extending into the cyst, were the two vasa deferentia. The stalk of the cyst was traced down to the prostatic urethra, where its
Fm. 3. Same as figure 2.
Lateral view
Fm. 4. Photograph of surgical specimen of Mullerian duct cyst showing ventral surface. V-vas; SY-seminal vesicle; P-prostate; C-connection of Mullerian duct cyst with posterior urethra.
attachment was divided. The cyst was removed in toto with most of the prostate, both seminal vesicles, and the distal 3 inches of the vasa deferentia (fig. 4). Pathological Report:~"The portion of the specimen representing the prostate
568
C. L. DEMING AND R. R. BERNEIKE
is traversed by a canal 7 mm. in diameter. This canal is continuous above with a large, somewhat flattened, oval-shaped cavity, the inner surface of which is grayish-white and composed of minute nodules and sacc11lations. No demonstrable connection exists between the lumen of the vasa and that of the cyst. Sections of prostate show the usual epithelial lining with ducts and acini. Sections of the large cyst wall itself show the wall to be made up of a thick layer of smooth muscle and fibrous connective tissue. It is partially lined by a flattened layer of deep blue staining epithelial cells. Diagnosis :~Mullerian duct cyst, seminal vesicles, and portions of prostate and vasa deferentia." The patient's convalescence was complicated by drainage of urine from the suprapubic wound for 4 days, but this responded satisfactorily to an inlying urethral catheter. A superficial wound infection was controlled by conservative measures, and the patient was discharged on the twenty-fourth postoperative day. The patient has been seen twice since he left the hospital, and he is free of urinary symptoms. Rectal examination two months after the patient's discharge from the hospital was normal except that in the region of the prostate there was palpable a mass of indurated tissue about two-thirds the size of the normal prostate. CONCLUSION
An attempt has been made to establish some clear clinical features diagnostic of Mullerian duct cyst. A detailed report of a case is presented.
789 Howard Ave., New Haven, Conn. REFERENCES BEGG, R. CAMPBELL: Massive cystadenoma of Muller's duct. Brit. J. Urol., 8: 1936. CoPPRIDGE, W. M.: Mi.illerian duct cysts. South. M. J., 32: 248-251, 1939. DEMING, CLYDE L.: Cyst of the seminal vesicle. Trans. Am. Assoc. Genito-Urinary Surg., 1935. EMMETT, JoHN L., AND BRAASCH, WILLIAM F.: Cysts of the prostate gland. J. Urol., 36: 236-249, 1936. HALLOCK, L. A.: Large hemorrhagic cyst of the prostate gland. Am. J. Cancer (Supp.) 15: 2331-2340, 1931. HENNESSEY, RusSELL A.: Mi.illerian duct cysts. J. Urol., 42: 1042-1050, 1939.